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11/38. Spindle epithelial tumor with thymus-like differentiation (SETTLE) of the thyroid.

    BACKGROUND: Tumors of the so-called intrathyroidal epithelial thymoma type are a rare group of thyroid neoplasm tumors. Of this type of tumor, spindle epithelial tumor with thymus like differentiation (SETTLE) has been reported only 17 times in English literature. methods: An 18-year-old woman was initially seen with a 6-cm left thyroid mass that was resected with a left thyroidectomy. Histopathologic features of the excised left thyroid tumor together with an immunohistochemistry and electron microscopy led us to a diagnosis of SETTLE. RESULTS: Most of the specimen was composed of highly cellular spindle cells that formed intersecting and streaming fascicles. The spindle cells showed both vimentin immunoreactivity and cytokeratin immunoreactivity but no immunoreactivity for thyroglobulin or calcitonin. Electron microscopic examination of the spindle cells demonstrated prominent cytoplasmic tonofilaments, desmosomes, and basal lamina consistent with epithelial cell origin. dna content analysis by flow cytometry revealed dna diploidy. CONCLUSIONS: SETTLE of thyroid gland is an extremely rare entity. A review of the literature reveals that SETTLE has distinctive morphologic features and an immunohistochemical profile.
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ranking = 1
keywords = neoplasm
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12/38. Epithelial-myoepithelial carcinoma of the lung. A case report and review of the literature.

    Primary lung tumors mimicking the salivary gland-type neoplasms are extremely rare. These types of tumors originate from submucosal bronchial glands. Epithelial-myoepithelial carcinoma is an uncommon tumor in this group, and only 7 cases have been reported. It is considered to be a low-grade malignant neoplasm. We report a case of epithelial-myoepithelial carcinoma of bronchial gland origin in a 73-year-old man who presented with coughing and dyspnea. The bronchus of the left lower lobe contained a tumor mass that had a polypoid growth pattern. The tumor also extended into the pulmonary parenchyma, forming a well-circumscribed mass with a pushing margin. The tumor consisted of epithelial and myoepithelial cells. The epithelial cells were positive for cytokeratins and epithelial membrane antigen, while the myoepithelial cells were positive for S100 protein and muscle-specific actin. According to these findings, we diagnosed epithelial-myoepithelial carcinoma. After undergoing pneumonectomy, the patient has been disease free for 34 months.
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ranking = 2
keywords = neoplasm
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13/38. A case of porocarcinoma from pre-existing hidroacanthoma simplex: need of early excision for hidroacanthoma simplex?

    BACKGROUND: Hidroacanthoma simplex (HAS) is an uncommon eccrine neoplasm of the skin. It is a benign neoplasm, but some cases of malignant change were reported in the literature. OBJECTIVE: To describe a rare case of porocarcinoma in a relatively short period from pre-existing HAS on the abdomen. methods: Wide local excision was performed on this neoplasm. RESULTS: There was no recurrence for 72 months after wide excision. CONCLUSION. : We recommend an early excision and careful attention in the case of HAS because of its potential malignant change.
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ranking = 3
keywords = neoplasm
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14/38. Imprint cytology of metastatic sialoblastoma. A case report.

    BACKGROUND: Sialoblastoma is a rare, aggressive and potentially malignant perinatal/congenital tumor that recapitulates the developing salivary gland. There is only 1 brief description of the cytologic findings of metastatic sialoblastoma and 1 poorly documented case of lung metastasis in the literature. CASE: A 75-month-old girl with a history of recurrent sialoblastoma initially diagnosed at 21 months and treated with multiple incomplete surgical excisions, chemotherapy and radiation presented with a solitary lung nodule. Imprint smears and frozen section of the mass were diagnostic of metastatic sialoblastoma. CONCLUSION: Cytologic features of sialoblastoma showed complete concordance with histology and included the presence of variably arranged, tight, solid clusters of atypical-appearing, basaloidlike cells in a background of dispersed epithelial and myoepithelial cells. The clusters contained admixed benign ductal cells and dense, metachromatic, magenta hyaline globular material with smooth, rounded outlines. The differential diagnoses include neoplasms composed of either basaloid cells and/or admixed hyaline matrix material and included pleomorphic adenoma, basal cell adenoma and adenoid cystic carcinoma. All these neoplasms affect patients in the first 2 years of life, whereas sialoblastoma usually occurs in the first 2 decades of life.
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ranking = 2
keywords = neoplasm
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15/38. Low-grade renal epithelial tumor originating from the distal nephron.

    There are few published reports of low-grade renal epithelial tumor originating from the distal nephron. However, it should not be disregarded clinically, because the actual number of patients with such tumors may be higher than expected. We investigated the immunohistochemical profile of a histologically distinct subtype of such a tumor in detail, in addition to the clinical course and imaging studies. The present study demonstrated that both glandular and spindle cell components of this tumor have a persistent characteristic of an epithelial tumor arising from the distal tubule or collecting duct. This tumor is a benign complex neoplasm that can be treated successfully with radical surgery. Beta-catenin and E-cadherin are suggested to play a crucial role in tumorigenesis and the biphasic arrangement of this neoplasm, concerning the expression of epithelial membrane antigen and carbohydrate antigen 19-9. We suggest that the term 'distal nephron epithelioma' is appropriate for classifying such rare but clinicopathologically distinct tumors.
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ranking = 2
keywords = neoplasm
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16/38. Malignant PEComa of the skull base.

    Perivascular epithelioid cell tumors (PEComas) are rare, usually benign lesions comprising a family of neoplasms including angiomyolipoma, lymphangioleiomyomatosis, clear cell "sugar" tumors, and clear cell myomelanocytic tumors. This report describes an apparent case of a malignant PEComa of the skull base in a 49-year-old woman, a previously undescribed location for this lesion.
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ranking = 1
keywords = neoplasm
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17/38. Carcinoma of the esophagus with mixed basaloid squamous and glandular differentiation: a distinct histological presentation.

    Esophageal cancer is the third most common gastrointestinal cancer and ranks among the ten commonest cancers worldwide. Histologically, approx 60% of esophageal cancers are adenocarcinomas and 40% are squamous cell carcinomas (SCC). Other rare cancers of the esophagus include small-cell carcinomas, squamous cell carcinomas with sarcomatous features, adenoid cystic carcinomas, and mucoepidermoid carcinomas. Basaloid squamous cell carcinoma (BSCC) or basaloid squamous carcinoma (BSC) is a distinct clinicopathological entity, seen more frequently in elderly males. Stage at presentation is often advanced and regional adenopathy or distant metastases are not uncommon at presentation. We describe an unusual case report of esophageal BSCC with glandular differentiation. The clinical significance of glandular differentiation in this rare type of tumor is not known.
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ranking = 0.081452264563002
keywords = cancer
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18/38. Epithelial-myoepithelial carcinoma of the base of tongue: pathology and management.

    Epithelial-myoepithelial carcinoma is a rare tumor which makes up about 0.2% of epithelial neoplasms of the salivary glands; parotid gland being the most common primary site of origin. The tumor may also very rarely originate in minor salivary glands of the base of the tongue. Due to rarity of its occurrence, histogenesis and clear cut therapeutic guidelines are not defined. The present report describes the case of a 48 year old male who was diagnosed to have a tubular variant of epithelial-myoepithelial carcinoma of the base of tongue, Stage T3 N0 M0 (Stage group III). The patient was treated with neoadjuvant chemotherapy followed by radical radiotherapy (Rt) and is alive with no evidence of disease 14 months following end of treatment.
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ranking = 1
keywords = neoplasm
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19/38. Mixed epithelial and stromal tumor of the kidney in a 12-year-old girl.

    Mixed epithelial and stromal tumor of the kidney (MESTK) is a rare kidney neoplasm that almost exclusively occurs in perimenopausal women, and long-term estrogen replacement is relevant to its pathogenesis. Herein is described an atypical case of MESTK uncovered in a 12-year-old premenarcheal girl without a history of prior estrogen use. On surgical specimen it was found that the well-circumscribed tumor measuring 14 cm arose from the lower pole of the right kidney, showing solid and fibrous-cystic areas. Microscopically, it was composed both of epithelial structures similar to renal tubules and stroma comprising non-specific spindle cells. Some intratumoral tubules showed affinities to distal-nephron-specific lectins, and those immunoreactive for proximal-tubule-specific CD15 were also present. In addition, primitive ductal structures were reactive both for CD15 and lectins, but immature epithelial elements typical of nephroblastoma were absent. Spindle cells were positive for actin, desmin and vimentin, and expressed progesterone and estrogen receptors. The tumor was comparable with MESTK, although some epithelia were associated with the immunophenotype of proximal tubules. The patient was free of disease postoperatively for 40 months. In the present case, remnants of the primitive periductal mesenchyme might be promoted to neoplastic cells by a sex-steroid surge during puberty.
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ranking = 1
keywords = neoplasm
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20/38. Composite glandular and endocrine tumors of the stomach with pancreatic acinar differentiation.

    Composite tumors of the stomach consisting of mixed glandular and endocrine components are rare. We report 3 cases of composite glandular and endocrine tumors with pancreatic acinar differentiation in the stomach with their clinicopathologic findings. The patients' presenting symptoms were variable and included abdominal pain, gastrointestinal hemorrhage, and weight loss. One patient with abdominal pain also had an elevated serum lipase level, clinically mimicking acute pancreatitis. The histology of these tumors was similar. They showed admixture of well-differentiated endocrine components with acinar and glandular components. The glandular component consisted of columnar epithelial cells resembling gastric foveolar or intestinal goblet cells, consistent with a well-differentiated adenocarcinoma. A panel of histochemical and immunohistochemical stains was performed, which included PAS, alcian blue, Mib1, CEA, cytokeratin 7, cytokeratin 20, Muc2, Muc5AC, chromogranin, synaptophysin, trypsin, chymotrypsin, lipase, insulin, gastrin, serotonin, and pancreatic polypeptide. While the immunoreactivity for cytokeratin 7, cytokeratin 20, Muc2, Muc5AC, and CEA was largely restricted to the glandular component, the endocrine and pancreatic acinar markers showed marked variability and overlap. All cases showed immunoreactivity for at least one of the exocrine pancreatic enzymes, and all expressed endocrine differentiation. Some degree of amphicrine differentiation was suggested in all cases. Two cases showed metastases in perigastric lymph nodes, which histologically resembled the primary tumor. In summary, these tumors represent another distinct type of composite glandular and endocrine gastric neoplasm with pancreatic acinar differentiation.
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ranking = 1
keywords = neoplasm
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